[Show abstract][Hide abstract] ABSTRACT: Background
Hypertrophic cardiomyopathy (HCM) is a primary myopathic process in which regional left ventricular dysfunction may exist without overt global left ventricular dysfunction. In obstructive HCM patients who underwent surgical myectomy (SM), we sought to determine if there is a significant association between echocardiographic longitudinal strain, histopathology, and in vitro myocardial performance (resting tension and developed tension) of the surgical specimen.
Methods and Results
HCM patients (n=122, 54±14 years, 54% men) undergoing SM were prospectively recruited. Longitudinal systolic strain and diastolic strain rates were measured at that basal septum (partially removed at SM) by using velocity vector imaging on preoperative echocardiography. Semiquantitative histopathologic grading of myocyte disarray and fibrosis and in vitro measurements of resting tension and developed tension were made in septal tissue obtained at SM. Mean basal septal systolic strain and diastolic strain rate were −8.3±5% and 0.62±0.4/s, while mild or greater degree of myocyte disarray and interstitial fibrosis were present in 85% and 87%, respectively. Mean resting tension and developed tension were 2.8±1 and 1.4±0.8 g/mm2. On regression analysis, basal septal systolic strain, diastolic strain rate, disarray, and fibrosis were associated with developed tension (β=0.19, 0.20, −0.33, and −0.40, respectively, all P<0.01) and resting tension (β=0.21, 0.22, −0.25, and −0.28, respectively, all P<0.01).
In obstructive HCM patients who underwent SM, left ventricular mechanics (echocardiographic longitudinal systolic strain and diastolic strain rates), assessed at the basal septum (myocardium removed during myectomy) and histopathologic findings characteristic for HCM (disarray and fibrosis) were significantly associated with in vitro myocardial resting and developed contractile performance.
Journal of the American Heart Association 10/2014; 3(6). DOI:10.1161/JAHA.114.001269 · 2.88 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Inflammatory diseases of the aorta, other than those of known infective etiology, are poorly understood. We analyzed a large series of affected patients who had histologic diagnoses with a view to improving the classification of the extent of aortitis to enable a more targeted approach of treatment.
Journal of Thoracic and Cardiovascular Surgery 08/2014; 149(2). DOI:10.1016/j.jtcvs.2014.08.003 · 3.99 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Objective
Inflammatory diseases of the aorta, other than those of known infective etiology, are poorly understood. We analyzed a large series of affected patients who were histologically diagnosed with a view to improving the classification of the extent of aortitis to enable a more targeted approach of treatment.
Between 1996 and 2012, we operated on 7,551 patients with ascending or aortic arch disease, of whom 279 had clinically diagnosed inflammatory disease and of these, 156 (2%) were found to have aortitis proven on histology. The study was approved by the Institutional Review Board of the Cleveland Clinic, with patient consent waived.
Patients were divided into four histologically based groups: Giant Cell Aortitis 31% (49/156), Takayasus Disease 5.1% (8/156), Isolated Aortitis 59% (92/156) and other 4.5% (7/156). Patterns of anatomical extent were also analyzed and, in particular, it was noted that Giant Cell and Isolated Aortitis had more extensive disease. Also specimen analysis suggested early indications of unrecognized pre-existent infections. Death after surgery occurred in 3.2% (5/156) and stroke in 1.9 % (3/156). Kaplan-Meier survival at 8 years was 55%. We present a classification for disease extent and management.
Aortitis continues to be a conundrum but good results are achievable with surgery based on a clearer understanding of the histological pattern and extent of disease that helps in subsequent targeted disease management.
[Show abstract][Hide abstract] ABSTRACT: Objectives:
Autoimmune aortitis is an under-recognized cause of ascending aorta disease
requiring surgery. Little is known about surgical outcomes and natural history
thereafter. Objectives of this study were to characterize surgical patients and
assess early and late outcomes.
From 1996 to 2012, 7,551 patients underwent ascending aorta surgery, 156 (2%) were
pathologically proven to have aortitis. Mean age was 66±14 years, and 64% were
women. Mean maximum preoperative diameters were 54±11 mm in the ascending aorta
and 41±15 mm in the descending aorta. Indications for surgery were degenerative
aneurysm (n= 132), acute aortic syndrome (n=15), aortic rupture (n=2) and
chronic dissection (n=8). Surgical resection extended beyond
the ascending aorta in 64% and Stage I elephant trunk (ET) was performed in 47
patients. Concomitant cardiovascular procedures included aortic valve
replacement (n=65), aortic valve repair (n= 21) and CABG (n=27). Mean follow-up
was 4.3±3.2 years.
Aortitis was classified as giant cell (n=49), Takayasu (n=8), isolated aortitis
(n=92), and aortitis due to other systemic disease (n=7). Diagnosis was made
preoperatively in 18% and postoperatively in 82%. Operative mortality was 3%,
and prevalence of stroke 1.9%, dialysis 1.9%, and reoperation for bleeding
5.1%. Postoperative immunosuppressant medications were used in 56 patients, 15
of whom underwent re-intervention. Freedom from death and re-intervention at 1,
5 and 8 years was 76.6%, 58.8% and 40.2 %.( Figure). There were no significant
differences in survival and aortic re-intervention among aortitis groups.
Autoimmune aortitis is most often diagnosed by postoperative histology, and
distal aortic involvement is common at presentation. Despite good surgical
outcomes, risk of aortic reoperation is high. Prophylactic ET construction
should be considered during ascending aorta repair, and close imaging follow-up is recommended.
[Show abstract][Hide abstract] ABSTRACT: Hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy, disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia, which contribute to disease progression. Longitudinal systolic and early diastolic strain rate (SR) measurements by speckle tracking echocardiography are sensitive markers of regional myocardial function. We sought to determine the association between septal SR and histopathologic findings in symptomatic HCM patients who underwent surgical myectomy.
We studied 171 HCM patients (documented on histopathology) who underwent surgical myectomy to relieve left ventricular outflow tract obstruction. Various clinical and echocardiographic parameters were recorded. Segmental longitudinal systolic and early diastolic SRs (of the septal segment removed at myectomy) were measured from apical 4- and 2-chamber views (VVI 2.0; Siemens, Erlangen, Germany). Histopathologic myocyte hypertrophy, disarray, small intramural coronary arteriole dysplasia, and interstitial fibrosis were classified as none, mild (1%-25%), moderate (26%-50%), and severe (>50%).
The mean age was 53 ± 14 years (52% men, ejection fraction 62% ± 5%, mean left ventricular outflow tract gradient 102 ± 39 mm Hg, and basal septal thickness of 2.2 ± 0.5 cm). Mean longitudinal systolic and early diastolic SRs were -0.91 ± 0.5 and 0.82 ± 0.5 (1/s), respectively. There was an inverse association between systolic and early diastolic septal SR and degree of myocyte hypertrophy, disarray, and interstitial fibrosis (all P < .05). There was no association between histopathologic characteristics and other echocardiography parameters. On multivariable regression analysis, myocyte disarray and echocardiographic septal hypertrophy were associated with systolic and early diastolic septal SR (P < .05).
In HCM patients, there is inverse association between various histopathologic findings and septal SR. Strain rate might potentially provide further insight into HCM pathophysiology.
American heart journal 09/2013; 166(3):503-11. DOI:10.1016/j.ahj.2013.06.011 · 4.56 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A 38-year-old woman with clinical T3N1M0 esophageal adenocarcinoma received an institutional protocol of induction chemotherapy, esophagectomy, and consolidation chemoradiotherapy. Three months after treatment, she had an acute mental status change develop and grand mal seizures. Intracranial imaging demonstrated massive cerebral air emboli, and a rapidly fatal neurologic deterioration ensued. At autopsy, a nonmalignant 0.5-cm gastric conduit to the left atrial fistula was identified. This case illustrates a rare, but fatal, late benign complication of aggressive therapy for locally advanced esophageal cancer.
The Annals of thoracic surgery 11/2011; 92(5):1901-3. DOI:10.1016/j.athoracsur.2011.03.054 · 3.65 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We sought to assess the diagnostic accuracy and incremental prognostic value of delayed hyper-enhancement cardiac magnetic resonance (DHE-CMR) compared with electrocardiographic and transthoracic echocardiographic (TTE) parameters in such patients.
Utility of DHE-CMR in the diagnosis of patients with suspected cardiac amyloidosis (CA) has recently been demonstrated, but its incremental prognostic utility is unclear.
Forty-seven consecutive patients (mean age 63 years, 70% men, 55% New York Heart Association functional class >II) with suspected CA who underwent electrocardiography (ECG), TTE, DHE-CMR, and biopsy (38 endomyocardial, 9 extracardiac) were studied. Low voltage on ECG was defined as S-wave in lead V(1) + R-wave in lead V(5) or V(6) <15 mm. TTE parameters, including deceleration time, E/E' ratio, and diastolic grade were recorded. CMR was considered positive with diffuse DHE of the subendocardium extending to adjacent myocardium. All-cause mortality was ascertained.
In the study population, 59% had low voltage on ECG, 30% had abnormal deceleration time < OR = 150 ms, 38% had E/E' ratio >15, and 47% had advanced (pseudonormal or restrictive) diastology.The diagnostic accuracy of DHE-CMR in patients undergoing endomyocardial biopsy was as follows: sensitivity 88%, specificity 90%, positive predictive value 88%, and negative predictive value 90%. On multivariable logistic regression testing of the diagnostic ability of various noninvasive imaging parameters, only DHE-CMR was significant (Wald chi-square statistic 9.6, p < 0.01). At 1-year post-biopsy, there were 9 (19%) deaths. On Cox proportional hazards analysis, only positive DHE-CMR was a predictor of 1-year mortality (Wald chi-square statistic 4.91, p = 0.03).
A characteristic DHE-CMR pattern is more accurate for diagnosis and is a stronger predictor of 1-year mortality in patients with suspected CA as compared with other noninvasive parameters.
[Show abstract][Hide abstract] ABSTRACT: In hypertrophic cardiomyopathy (HCM) patients undergoing surgical myectomy, we sought to determine the association between pre-operative cardiac magnetic resonance (CMR) findings, small intramural coronary arteriole dysplasia (SICAD) on histopathology, and ventricular tachycardia (VT).
Myocardial scarring (fibrosis) and SICAD are frequently observed on histopathology in HCM patients. CMR measures wall thickness and detects scar.
Sixty symptomatic HCM patients (62% men; mean age 51 +/- 14 years), with preserved ejection fraction (mean 64 +/- 5%) and no angiographic coronary disease underwent CMR (cine and delayed post-contrast) using a Siemens 1.5 T scanner, followed by septal myectomy. Maximal basal septal thickness was recorded on cine CMR. Scar was determined (percentage of total myocardium) on delayed post-contrast CMR images and quantified as none, mild (0% to 25%), moderate (26% to 50%), or severe (>50%). VT was assessed using Holter monitoring. Degree of SICAD was determined (normal, mild, moderate, and severe) on histopathology of surgical specimen.
SICAD and scar were seen in 45 (75%) and 38 (63%) patients, respectively. In 15 patients without SICAD, 12 (80%) had no scar; 23 (70%) patients with mild SICAD had mild scar on CMR. On multivariate analysis, degree of SICAD was independently associated with scar on CMR (Wald chi-square statistic: 6.8, p < 0.01). Patients with basal septal scar on CMR had higher VT frequency compared with those without (27% vs. 5%, p = 0.03).
A strong association exists between degree of SICAD and myocardial scarring seen on CMR.
Journal of the American College of Cardiology 07/2009; 54(3):242-9. DOI:10.1016/j.jacc.2009.04.026 · 15.34 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Cardiac amyloidosis (CA) is generally associated with a poor prognosis and significantly increased mortality. We sought to identify predictors of longer-term survival in patients with endomyocardial biopsy (EMB)-documented CA. Forty-five consecutive patients with EMB-documented CA were studied from January 1998 to December 2003. Age, gender, New York Heart Association class, medications, presence of light-chain amyloid, and electrocardiographic voltage were recorded. Baseline left ventricular (LV) ejection fraction, deceleration time, diastolic function, LV mass, ventricular septal thickness, and myocardial performance index ([isovolumic contraction time + isovolumic relaxation time]/ejection time) were recorded. Mean age was 66 +/- 10 years with 34 men (76%). New York Heart Association class >II was noted in 26 patients (58%) and low voltage on electrocardiogram (S wave [lead V(1)] + R wave [lead V(5)] < or =15) in 12 (27%). Mean LV ejection fraction, ventricular septal thickness, and LV mass were 46 +/- 13%, 1.7 +/- 0.42 cm, and 303 +/- 114 g, respectively. Deceleration time < or =150 ms was found in 19 (42%) and myocardial performance index >0.6 in 15 (33%). At a median follow-up of 1.7 years, there were 25 deaths (56%). On univariate Kaplan-Meier analysis, New York Heart Association class >II, deceleration time <150 ms, and beta-blocker use were associated with increased mortality (log-rank statistic p values <0.001, <0.05, and 0.01, respectively). On Cox proportional hazard survival analysis, only New York Heart Association class was significantly associated with increased mortality (hazard ratio 3.92, 1.92 to 7.95, p = 0.0002). In conclusion, in patients with EMB-documented CA, longer-term survival is more strongly associated with New York Heart Association functional class compared with electrocardiographic and echocardiographic variables.
The American journal of cardiology 06/2009; 103(10):1429-33. DOI:10.1016/j.amjcard.2009.01.361 · 3.43 Impact Factor